CPK Levels

This morning Isa had a real bad episode. She awoke with a blood sugar of 29. After treatment it went up to 42 (15 minutes). Following more treatment and arrival at the doctor’s it was 203. While we were there our pediatrician showed us the letter that our metabolic specialist had sent her. In the letter it said that Isa’s FOD is a possible VLCAD (later changed to LCHAD). Also the letter indicated that at her last doctor’s appointment she had elevated CPK levels. What is CPK? 

Sharon 

LCHAD mom

CPK stands for creatine (phospho) kinase, sometimes also referred to as “c k” level. It measures enzymes in the blood from muscle breakdown and there is an extended cpk that detects isoenzymes that tells if it is coming from heart muscle or skeletal muscle. Rachel’s has always been totally muscular, although if her body is not getting what it wants and enough of it (high cpk alerts me to this), it can be very damaging to her heart since this is one of the effects of the particular phenotype of the disease that she has. We routinely check her CPK when she is sick. With illness or infection the metabolic rate speeds up and with VLCAD, inadequate calories can lead to muscle breakdown. We also have checked it when she is well to see what her “normal” average level runs (lab norm 0-225).

Lisa 

VLCAD mom

Our kids’ CPKs rise every time they get an illness. It was explained to me in laymen’s terms like this: Your body is like an engine. It gives off exhaust just like an engine. The CPK level is the level of exhaust caused by the breakdown of muscle in your body. Normal levels for a toddler is 50-300. When an FOD child gets ill, they use all the sugar fuel first then reverts to the breakdown of skeletal muscle for energy. This causes the rise in the CPK (exhaust) levels.

Justin’s high has been 87,000. Autumn’s high 39,000. They always go back to normal. Originally the heart was a concern, but we have since ruled that out since the breakdown is from their skeletal muscles only. They treat it with sodium bicarbonate IV. I think the key to keeping the CPK levels down is keeping the sugar levels high and increasing the fluids. The main concern of high CPK levels is kidney damage. The kidneys are responsible for cleaning the blood and if there is a lot more “exhaust” in the blood it really puts a strain on the kidneys and can cause some permanent damage. This may not be technically correct in all aspects, but that is how it was explained to us.

Kim 

LCHAD mom x 2